Institute of liver diseases and transplant, MGM Healthcare, Multi Super Speciality Hospital in Chennai, has successfully treated a 35-year-old male from Jordan with a rare genetic blood disease – Paroxysmal Nocturnal Hemoglobinuria (PNH) (recurrent propensity for clotting and lysis of blood) and Budd-chiari syndrome (BCS) a disorder characterised by obstruction of hepatic venous outflow and inferior vena cava reaching up to heart and end stage liver failure.
Analysing the complexities involved in this case, the experts from MGM Hospital took a multidisciplinary approach, including liver transplant surgeons (Dr Thiagarajan Srinivasan, Director, Liver Transplant, Dr Karthik Mathivanan, Associate Director Liver Transplant), cardio thoracic surgeons(Dr KR Balakrishnan, Chairman, Heart Transplant) haematologists, liver anaesthetists and critical care specialists( Dr Dinesh Babu and Dr Nivash C) and hepatologists devised a unique treatment protocol for the patient which included a combination of a liver transplant and removal of clots from the blood vessels reaching the heart.
Explaining the treatment procedure, Dr Thiagarajan Srinivasan said, “The patient’s brother donated a part of his liver after ascertaining the absence of genetic disease. Before and after surgery, the patient underwent multiple courses of monoclonal antibody therapy(Eculuzimab) to keep the clotting and hemolysis at bay. A combined living donor liver transplant with replacement of the inferior vena cava with a synthetic Dacron graft* was done. He added that it has been four months since the procedure and the patient was doing well. The team of doctors are planning a bone marrow transplant to cure his genetic illness. To our knowledge this is the first time in the country such a complex course of treatment with liver transplant has happened for such a complicated rare genetic disorder with liver failure.”
The patient was suffering from a rarest-of-rare fatal, sharing his thoughts, Dr. Karthik Mathivanan said, “Genetic condition which was leading to end stage liver failure, the patient needed a liver transplant with replacement of inferior vena cava. It was the only life-saving option, though it posed very high chances of recurrent clotting of blood vessels.”
Doctors Dinesh and Nivash said, “Rapid depletion of red blood cells, blood clot formation, impaired bone marrow function was treated with monoclonal antibody therapy and effective anticoagulants. The team of skilled specialists at MGM have given a new lease of life to the 35-year-old man”.
*Dacron graft is a synthetic polyester material used to replace body tissues. They are in the shape of a tube to replace or repair blood vessels.
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